The BSE ongoing surveillance program will sample approximately 40,000 animals each year. Under the program, USDA will continue to collect samples from a variety of sites and from the cattle populations where the disease is most likely to be detected, similar to the enhanced surveillance program procedures.

WASHINGTON, July 20, 2006-Agriculture Secretary Mike Johanns announced today that the U.S. Department of Agriculture will soon begin transitioning to an ongoing Bovine Spongiform Encephalopathy (BSE) surveillance program that corresponds to the extremely low prevalence of the disease in the U.S.

"It's time that our surveillance efforts reflect what we now know is a very, very low level of BSE in the United States," said Johanns. "This ongoing surveillance program will maintain our ability to detect BSE, provide assurance that our interlocking safeguards are successfully preventing BSE, while continuing to exceed science-based international guidelines."

The ongoing BSE surveillance program will sample approximately 40,000 animals each year. Under the program, USDA will continue to collect samples from a variety of sites and from the cattle populations where the disease is most likely to be detected, similar to the enhanced surveillance program procedures.

The new program will not only comply with the science-based international guidelines set forth by the World Animal Health organization (OIE), it will provide testing at a level ten times higher than the OIE recommended level.

USDA has an obligation to provide 30 days notice of the change to contractors who are performing the sampling and testing, so the earliest the new surveillance program would begin is late August. Once the ongoing surveillance program begins, USDA will periodically analyze the surveillance strategy to ensure the program provides the foundation for market confidence in the safety of U.S. cattle.

In April, USDA released an analysis of 7 years of BSE surveillance data. This included data from an enhanced surveillance program, which began in June 2004, as a one-time effort to determine the prevalence of BSE in the United States. The analysis concluded that the prevalence of BSE in the United States is less than 1 case per million adult cattle. The analysis further revealed that the most likely number of cases is between 4 and 7 infected animals out of 42 million adult cattle. The analysis was submitted to a peer review process and a panel of outside experts affirmed the conclusions.

The enhanced surveillance program has been funded using emergency CCC funds totaling $157.8 million since June 2004. Ongoing surveillance will cost approximate $17 million per year using funds appropriated by Congress. The President's FY 2007 budget request includes this level of funding.

BSE surveillance is not a food safety program. Human and animal health is protected by a system of interlocking safeguards, including the removal of specified risk materials - those tissues that studies have demonstrated may contain the BSE agent in infected cattle, along with the U.S. Food and Drug Administration's 1997 ruminant to ruminant feed ban. Scientific studies indicate that the longer a feed ban is in place, the lower the prevalence of BSE will become.

An outline of the ongoing BSE surveillance plan is available at http://www.aphis.usda.gov/newsroom/hot_issues/bse.shtml.

The U.S. Department of Agriculture was quick to assure the public earlierthis week that the third case of mad cow disease did not pose a risk tothem, but what federal officials have not acknowledged is that this latestcase indicates the deadly disease has been circulating in U.S. herds for atleast a decade.

The second case, which was detected last year in a Texas cow and which USDAofficials were reluctant to verify, was approximately 12 years old.

These two cases (the latest was detected in an Alabama cow) present apicture of the disease having been here for 10 years or so, since it isthought that cows usually contract the disease from contaminated feed theyconsume as calves. The concern is that humans can contract a fatal,incurable, brain-wasting illness from consuming beef products contaminatedwith the mad cow pathogen.

"The fact the Texas cow showed up fairly clearly implied the existence ofother undetected cases," Dr. Paul Brown, former medical director of theNational Institutes of Health's Laboratory for Central Nervous SystemStudies and an expert on mad cow-like diseases, told United PressInternational. "The question was, 'How many?' and we still can't answerthat."

Brown, who is preparing a scientific paper based on the latest two mad cowcases to estimate the maximum number of infected cows that occurred in theUnited States, said he has "absolutely no confidence in USDA tests beforeone year ago" because of the agency's reluctance to retest the Texas cowthat initially tested positive.

USDA officials finally retested the cow and confirmed it was infected sevenmonths later, but only at the insistence of the agency's inspector general.

"Everything they did on the Texas cow makes everything USDA did before 2005suspect," Brown said. ...snip...end

''USDA does not know what's going on''. ''USDA is protecting the industry''. ''SHOULD the state of California step in''

Stanley Prusiner

''nobody has ever ask us to comment''

''they don't want us to comment''

''they never ask''

i tried to see Venemon, after Candian cow was discovered with BSE. went to see lyle. after talking with him... absolute ignorance... then thought i should see Venemon... it was clear his entire policy was to get cattle bonless beef prods across the border... nothing else mattered... his aids confirmed this... 5 times i tried to see Venemon, never worked... eventually met with carl rove the political... he is the one that arranged meeting with Venemon... just trying to give you a sense of the distance... healh public safety... was never contacted... yes i believe that prions are bad to eat and you can die from them...END

PLEASE NOTE, if spontaneous scrapie or CWD does not occur, then why is it that now only atypical BSE and sproadic CJD are capable of spontaneous mutation $$$ seems the UK study just posted about different strains of UK scrapie cause different strains of TSE when transmitted to UK cattle in the lab would dispute the spontaneous, natural, sporadic, lame duck excuse for any TSE, and how could these few 'unusual cases' _scientifically_ explain 85%+ of all sCJD, of which there are 6 documented phenotypes, or 5, depending whom you believe ? bottom line, they can't......TSS

Perspectives BIOMEDICINE: A Fresh Look at BSE Bruce Chesebro* Mad cow disease, or bovine spongiform encephalopathy (BSE), is the cattle form of a family of progressive brain diseases. These diseases include scrapie in sheep, Creutzfeldt-Jakob disease (CJD) in humans, and chronic wasting disease (CWD) in deer and elk. They are also known as either "prion diseases" because of the association of a misfolded cellular prion protein in pathogenesis or "transmissible spongiform encephalopathies" (TSEs) because of the spongelike nature of the damaged brain tissue (1).

The recent discovery of two BSE-infected cows, one in Canada and one in the United States, has dramatically increased concern in North America among meat producers and consumers alike over the extent to which BSE poses a threat to humans as well as to domestic and wild animals. The European BSE epidemic of the late-1980s seems to have been initiated a decade earlier in the United Kingdom by changes in the production of meat and bone meal (MBM) from rendered livestock, which led to contamination of MBM with the BSE infectious agent. Furthermore, the fact that UK farmers fed this rendered MBM to younger animals and that this MBM was distributed to many countries may have contributed to the ensuing BSE epidemic in the United Kingdom and internationally (2).

Despite extensive knowledge about the spread of BSE through contaminated MBM, the source of BSE in Europe remains an unsolved mystery (2). It has been proposed that BSE could be derived from a cross-species infection, perhaps through contamination of MBM by scrapie-infected sheep tissues (see the figure). Alternatively, BSE may have been an endemic disease in cattle that went unnoticed because of its low level of horizontal transmission. Lastly, BSE might have originated by "spontaneous" misfolding of the normal cellular prion protein into the disease-associated abnormal isoform (3), which is postulated to be the infectious agent or "prion."

Five possible sources of BSE in North American cattle. Sheep, deer, and elk could spread prion diseases (TSEs) to cattle through direct animal contact or contamination of pastures. Endemic BSE has not been proven to exist anywhere in the world, but it is difficult to exclude this possibility because of the inefficient spread of BSE infectivity between individual animals (2). BSE caused by spontaneous misfolding of the prion protein has not been proven. CREDIT: KATHARINE SUTLIFF/SCIENCE

Spontaneous protein misfolding is not a new phenomenon as proteins are known to sometimes misfold after synthesis. Cells in turn have devised ingenious ways to deal with this problem. These include molecular chaperone proteins that bind to misfolded proteins and help them to unfold, and organelles called proteosomes that degrade misfolded or unwanted proteins. However, although misfolded prion proteins have been generated in test tubes as well as in cultured cells, it has been difficult to demonstrate that such misfolded abnormal prion proteins are infectious (4, 5). Even the most recent data do not prove conclusively that infectivity has been generated in vitro because misfolded synthetic prion proteins were not able to transfer disease directly to wild-type mice (6). To obtain infectivity and subsequent prion disease, the misfolded proteins had to be inoculated and incubated for 1 to 2 years in transgenic mice that overexpressed a mutant version of the prion protein. Previous data from this group showed that transgenic mice expressing high amounts of prion protein developed neurological disease without inoculation of misfolded prion protein (7). Thus, at the biochemical level, the critical attributes of the misfolded prion protein required for infectivity are not known, and misfolding of prion protein alone may not be sufficient to generate an infectious agent (. Nevertheless, the idea that BSE might originate due to the spontaneous misfolding of prion proteins has received renewed interest in the wake of reports suggesting the occurrence of atypical BSE (9-11). These results imply that new strains of cattle BSE might have originated separately from the main UK outbreak. Where and how might such strains have originated? Although such rare events cannot be studied directly, any number of sources of the original BSE strain could also explain the discovery of additional BSE strains in cattle (see the figure). However, it would be worrisome if spontaneous BSE were really a valid etiology because such a mechanism would be impossible to prevent--unlike other possible scenarios that could be controlled by large-scale eradication of TSE-positive animals.

Another way to look at this problem is to examine evidence for possible spontaneous TSE disease in other animals besides cattle. Spontaneous BSE would be extremely difficult to detect in cattle, where horizontal spread is minimal. However, in the case of the sheep TSE disease, scrapie, which spreads from ewes to lambs at birth as well as between adults, spontaneous disease should be detectable as new foci of clinical infection. In the early 1950s scrapie was eradicated in both Australia and New Zealand, and the mainland of both these countries has remained scrapie-free ever since. This scrapie-free status is not the result of selection of sheep resistant to scrapie because sheep from New Zealand are as susceptible as their UK counterparts to experimental scrapie infection (12). These experiments of man and nature appear to indicate that spontaneous clinical scrapie does not occur in sheep. Similarly, because CWD is known to spread horizontally, the lack of CWD in the deer or elk of eastern North America but its presence in western regions would also argue against a spontaneous disease mechanism. This is particularly noteworthy in New Zealand, where there are large numbers of deer and elk farms and yet no evidence of spontaneous CWD. If spontaneous scrapie does not occur in sheep or deer, this would suggest that spontaneous forms of BSE and sporadic Creutzfeldt-Jakob disease (sCJD) are unlikely to be found in cattle or humans. The main caveat to this notion is that spontaneous disease may arise in some animal species but not others. In humans, sCJD--which is considered by some researchers to begin by spontaneous misfolding of the prion protein--usually takes more than 50 years to appear. Thus, in animals with a shorter life-span, such as sheep, deer, and cattle, an analogous disease mechanism might not have time to develop.

What can we conclude so far about BSE in North America? Is the BSE detected in two North American cows sporadic or spontaneous or both? "Sporadic" pertains to the rarity of disease occurrence. "Spontaneous" pertains to a possible mechanism of origin of the disease. These are not equivalent terms. The rarity of BSE in North America qualifies it as a sporadic disease, but this low incidence does not provide information about cause. For the two reported North American BSE cases, exposure to contaminated MBM remains the most likely culprit. However, other mechanisms are still possible, including cross-infection by sheep with scrapie or cervids with CWD, horizontal transmission from cattle with endemic BSE, and spontaneous disease in individual cattle. Based on our understanding of other TSEs, the spontaneous mechanism is probably the least likely. Thus, "idiopathic" BSE--that is, BSE of unknown etiology--might be a better term to describe the origin of this malady.

What does all this imply about testing cattle for BSE in North America? Current testing in the United States indicates that BSE is rare (one positive result in 40,000 cattle tested). However, additional testing of 200,000 head of slaughtered cattle over the next 1 to 2 years, as recently proposed by the U.S. Department of Agriculture (USDA), should tell us the incidence more precisely. Nevertheless, if any rare cases are detected, we may still not know their origin. If evidence arises of a focal occurrence of BSE, we might gain important insight into unexpected sources of contamination. However, because current tests do not seem to be able to detect BSE in infected animals less than 30 months of age, even more extensive testing will not completely guarantee the negative status of younger animals in the food chain. Therefore, the alternative option of testing all slaughtered cattle, as implemented in some countries such as Japan, would appear to provide little additional benefit. This fact has been acknowledged as the basis for a new agreement between the United States and Japan aimed at reestablishing the beef trade between the two countries.

One problem with the current U.S. testing program was the announcement a few months ago of unconfirmed positive BSE tests in two additional North American animals that were subsequently found to be negative when tested with the more accurate method of Western blotting. The public release of information about unconfirmed positive tests detected by the rapid test used for mass screening may be a good idea in the interest of openness, but it ha s the potential to create unwarranted anxiety. If unconfirmed positives are a frequent occurrence, it would seem reasonable to follow a more cautious approach and wait until confirmatory testing is complete before publicly announcing the details.

Based on the experience of many European countries, the mainstays of controlling BSE in cattle and avoiding spread to humans are threefold: first, eliminate feeding of ruminant tissues to ruminants; second, remove high-risk cattle tissues from human food; and third, continue to test for BSE in cattle in order to monitor progress with the elimination of the disease on a local and national basis. In the next 12 months, after extensive USDA test results are available, the extent of any possible BSE spread in the United States will be better documented. But, in fact, the United States and Canada have already instituted the most important steps to prevent the spread of cattle BSE in advance of the results--that is, a ban on feeding ruminant MBM to other ruminants and removal of high-risk tissues from meat for human consumption. It is hoped that the new data will not deviate enough from previous predictions to require further measures for management of this problem. The most important line of defense against any possible spread of BSE will be to maintain strict vigilance in the implementation of the current regulations.